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Clin J Am Soc Nephrol. 2009 Aug;4(8):1312-6. doi: 10.2215/CJN.01090209. Epub 2009 Jun 25.

Complement inhibitor eculizumab in atypical hemolytic uremic syndrome.

Author information

1
Department of Pediatrics, Medical University Graz, Auenbruggerplatz 30, Graz A-8036, Austria. christoph.mache@medunigraz.at

Abstract

BACKGROUND AND OBJECTIVES:

Atypical hemolytic uremic syndrome (aHUS) is associated with a congenital or acquired dysregulation of the complement alternative pathway that leads to continuous complement activation on host cells causing inflammation and damage. Eculizumab, a humanized mAb against complement protein C5, inhibits activation of the terminal complement pathway.

DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS:

We report an adolescent with relapsing unclassified aHUS. On admission, a high plasma creatinine level indicated a poor prognosis, and hemodialysis had to be started. Plasma exchanges were initially effective against the microangiopathic hemolytic activity and allowed a temporary improvement of renal function with termination of hemodialysis after 7 wk. Subsequently, plasma exchanges (three times per week) failed to prevent ongoing aHUS activity and progressive renal failure. After 12 wk, aHUS treatment was switched to eculizumab.

RESULTS:

Eculizumab was effective in terminating the microangiopathic hemolytic process in two aHUS relapses; however, after normalization of complement activity, aHUS recurred and ultimately led to anuric end-stage renal failure.

CONCLUSIONS:

In this patient, complement inhibition by eculizumab temporarily terminated the microangiopathic hemolytic activity. Nevertheless, renal damage as a result of preceding and subsequent aHUS activity resulted in end-stage renal failure; therefore, therapeutic success may depend on early administration of eculizumab. The optimal duration of treatment may be variable and remains to be determined.

PMID:
19556379
PMCID:
PMC2723971
DOI:
10.2215/CJN.01090209
[Indexed for MEDLINE]
Free PMC Article
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